Adrenocorticotropic Hormone Induced Status Dystonicus in a Child with West Syndrome (original) (raw)
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Comparison of two low dose ACTH therapies for West syndrome: their efficacy and side effect
Brain & Development, 2005
In order to clarify the appropriate usage of adrenocorticotropic hormone (ACTH), the efficacy and side effects of two different regimens of low dose ACTH therapy were compared. Thirty-four patients with West syndrome (WS) were treated with ACTH. The dose of synthetic ACTH-Z was 0.015 mg/kg/dose in 18 patients who were treated between 1996 and 1998 (regimen A), and 0.010 mg/kg/dose in 16 patients who were treated between 1999 and 2001 (regimen B). Patients were classified into cryptogenic and symptomatic groups. Efficacy and adverse effects of ACTH were compared between regimens A and B. Similar analyses were performed after stratification into cryptogenic and symptomatic groups. The efficacy of ACTH was not different between regimens A and B. However, among patients with symptomatic WS, the number of ACTH injections and the dose of ACTH until cessation of spasms were significantly smaller in regimen A than in regimen B. There was no significant difference in these variables between the regimens among those with cryptogenic WS. Adverse effects were not different between regimens A and B. 0.010 mg/kg per day of ACTH will be adequate for cryptogenic WS, but 0.015 mg/kg per day of ACTH is recommended for symptomatic WS. q
Response and Outcome of Moderate Dose Adrenocorticotropic Hormone in the Treatment of West Syndrome
Journal of National Institute of Neurosciences Bangladesh, 2022
Background: West Syndrome (WS) consist of a triad of epileptic spasms, hypsarrhythmia on EEG and arrest or regression of psychomotor development. Although ACTH has been found to be effective in the treatment of WS, questions remain regarding the optimum dosage, type of ACTH, duration of therapy, and its comparative efficacy with other treatment options. Objective: To assess the response and outcome of treatment with moderate dose (100 IU/m2) ACTH in children of west syndrome. Methodology: This was a prospective observational study and done over 1-year period (July 2017 to June 2018) in Pediatric Neurology OPD, National Institute of Neuroscience and Hospital (NINS&H), Dhaka among the patients with West syndrome. All study participants were treated with moderate dose ACTH (100 unit/m2) (I/M) and treatment completed within 12 weeks. Patients were followed up at 2, 4, 6 and 12 WKs of treatment. Results: Total 52 cases were for enrolled. In this study it was found that complete cessation...
Pediatric Neurology, 2015
OBJECTIVE: A single-center, single-blind, parallel-group, randomized clinical trial was performed to test the null hypothesis that adrenocorticotropic hormone is not superior to high-dose prednisolone for treatment of newly diagnosed West syndrome. METHODS: Newly diagnosed infants with West syndrome were randomized to receive 14 days of oral prednisolone (40-60 mg/day) or a synthetically prepared intramuscular long-acting adrenocorticotropic hormone (40-60 IU/every other day [0.5-0.75 mg]) according to the United Kingdom Infantile Spasm Study protocol. They were blindly evaluated for infantile spasm remission by day 14, electroclinical remission (spasm cessation þ resolution of hypsarrhythmia on a 30-minute electroencephalograph) by day 14 and continued spasm freedom for 28 days. RESULTS: Ninety-seven patients were enrolled in the study, with 48 of them receiving prednisolone and 49 receiving ACTH. There was no significant difference in the baseline characteristics or risk factors for the two treatment groups. By day 14, cessation of infantile spasms occurred in 28/48 (58.3%) infants on prednisolone compared with only 18/49 (36.7%) infants given adrenocorticotropic hormone (P ¼ 0.03) and electroclinical remission in 21 on prednisolone compared with nine on adrenocorticotropic hormone (P ¼ 0.007). Sustained spasm control for 28 consecutive days following electroclinical remission occurred in 15 children on prednisolone compared with six on adrenocorticotropic hormone (P ¼ 0.008). The total number of days required for spasm cessation was significantly less in those treated with prednisolone (3.85 days AE 2.4) compared with adrenocorticotropic hormone (8.65 days AE 3.7) (P ¼ 0.001). Among patients who did not achieve remission, there was a non-significant trend toward greater quantitative reduction of spasms with prednisolone than with adrenocorticotropic hormone (P ¼ 0.079). CONCLUSION: Synthetic adrenocorticotropic hormone of 40-60 IU/every other day did not yield superior rates of electroencephalographic or clinical remission when compared with prednisolone of 40-60 mg/day. Significantly, more patients achieved electroclinical remission when treated with prednisolone than with adrenocorticotropic hormone.
Corticotrophin-ACTH in Comparison to Prednisolone in West Syndrome – A Randomized Study
The Indian Journal of Pediatrics, 2018
Objective To compare the outcomes of adrenocorticotrophic hormone (ACTH) and Prednisolone therapy in children with West syndrome. Methods The study was done at a tertiary health centre for children. The pediatric neurologist at the centre enrolled children into the study based on the inclusion and exclusion criteria. They were evaluated in detail, classified according to etiologic type and then, randomly assigned into two treatment groups, either ACTH or Prednisolone. They were followed at regular intervals till 6 mo. Results There was no difference between ACTH and Prednisolone groups with respect to all the outcomes measured. Cessation of spasms was achieved in 6/15 (40%) in Prednisolone group and 9/18 (50%) in ACTH group (p = 0.3906). The average time for achieving cessation was 6.9 and 8 d in ACTH and Prednisolone groups respectively (p = 0.7902). The relapse rates were 18.18 and 50% in ACTH and Prednisolone groups respectively (p = 0.28). The side-effects profile, subsequent epilepsy rates and improvement in milestones were similar in both the treatment groups. Conclusions There is no significant difference in children treated with ACTH and Prednisolone. Study results cannot be generalized due to small sample size. However, Prednisolone can be a suitable alternative to ACTH in resource poor settings.
Pediatric Neurology, 2017
OBJECTIVE: We earlier completed a single-blind, parallel-group, randomized clinical trial to test the null hypothesis that adrenocorticotropic hormone (ACTH) is not superior to high-dose prednisolone for short-term control of West syndrome. We now present long-term follow-up data for spasm control for individuals who completed this earlier trial. METHODS: Infants with untreated West syndrome were randomized to receive 14 days of prednisolone (40 to 60 mg/day) or intramuscular long-acting ACTH (40 to 60 IU every other day). They were evaluated at three, six, and 12 months to evaluate long-term spasm control. RESULTS: The total number of infants treated was 97 (48 prednisolone; 49 ACTH). All completed the treatment course. Eighty-five, 82, and 76 children were available for followup at three, six, and 12 months. The number lost to follow-up at each interval was not statistically different. Likelihood of spasm freedom at three months was significantly higher for prednisolone (64.6%) than for ACTH (38.8%) (P = 0.01; odds ratio = 2.9; 95% confidence interval = 1.3 to 6.6). At six months (P = 0.19) and twelve months (P = 0.13), the control of spasms was not statistically different, although a trend in favor of prednisolone was documented at both of these time points (58.3% versus 44.9% for ACTH at six months and 56.2% versus 40.8% with ACTH at 12 months). After initial remission by day 14 (n = 46), the likelihood of a relapse within the next 12 months was not statistically different between the two treatment groups (P = 0.1). CONCLUSIONS: Control of spasms at three months was significantly better if initially treated with prednisolone. Control of spasms at six and 12 months was not significantly different despite a trend favoring prednisolone. Risk of relapse following initial remission was similar in the two groups.
Update on pediatric dystonias: etiology, epidemiology, and management
Dystonia is a movement disorder characterized by sustained muscle contractions producing twisting, repetitive, and patterned movements or abnormal postures. Dystonia is among the most commonly observed movement disorders in clinical practice both in adults and children. It is classified on the basis of etiology, age at onset of symptoms, and distribution of affected body regions. Etiology: The etiology of pediatric dystonia is quite heterogeneous. There are many different genetic syndromes and several causes of symptomatic syndromes. Dystonia can be secondary to virtually any pathological process that affects the motor system, and particularly the basal ganglia. Classification: The etiological classification distinguishes primary dystonia with no identifiable exogenous cause or evidence of neurodegeneration and secondary syndromes. Treatment: Treatment for most forms of dystonia is symptomatic and includes drugs (systemic or focal treatments, such as botulinum toxin) and surgical procedures. There are several medications including anticholinergic, dopamine-blocking and depleting agents, baclofen, and benzodiazepines. In patients with dopamine synthesis defects L-dopa treatment may be very useful. Botulinum toxin treatment may be helpful in controlling the most disabling symptoms of segmental or focal dystonia. Long-term electrical stimulation of the globus pallidum internum appears to be especially successful in children suffering from generalized dystonia.
Neuromuscular Disorders, 2017
Duchenne Muscular Dystrophy (DMD) is the most frequent muscular dystrophy in childhood, with a worldwide incidence of one in 5000 live male births. It is due to mutations in the dystrophin gene leading to absence of full-length dystrophin protein. Central nervous system involvement is well-known in Duchenne Muscular Dystrophy. The multiple dystrophin isoforms expressed in brain have important roles in cerebral development and functioning. The association of Duchenne Muscular Dystrophy with seizures has been reported, and there is a higher prevalence of epilepsy in Duchenne Muscular Dystrophy patients (between 6.3% and 12.3%) than in the general pediatric population (0.5-1%). Duchenne Muscular Dystrophy patients may present with focal seizures, generalized tonic-clonic seizures or absences. We report on two boys in whom Duchenne Muscular Dystrophy is associated with epileptic spasms and hypsarrhythmia that fulfil the criteria for West syndrome, thus extending the spectrum of seizure types described in Duchenne Muscular Dystrophy patients.
Polysomnographical assessment of the pathophysiology of West syndrome
Brain and Development, 2001
In this brief review, the sleep studies on patients with West syndrome (WS) were summarized. In addition to the previously reported common finding for sleep in WS-reduction of the amount of rapid-eye-movement (REM) sleep-weakness of phasic suppression of chin muscle activity in WS patients has recently been found. The degree of this weakness is quantified by the phasic inhibition index (PII), which has been found to reflect a patient's prognosis as to convulsions. PII is proposed to be a useful parameter for assessing the prognosis of WS. Since the pontine tegmentum is involved in the production of the REM-related phasic loss of muscle activity in REM sleep, WS patients are hypothesized to have a functional instability of the pontine tegmentum. After adrenocorticotropin (ACTH) treatment, PII decreased significantly in all WS patients examined. Taken together with the effects of corticosteriods on PII, and the incidence of phasic chin muscle activity in patients with congenital adrenal hyperplasia and nephrotic syndrome, ACTH is hypothesized to suppress the spasms in WS patients not only through corticosteroids, but also through a direct action on the pontine tegmentum. Since PII has been reported to be elevated in patients with an autistic tendency, the appearance of an autistic tendency is also hypothesized to be involved in the functional disturbance of the pontine tegmentum.
Journal of Epilepsy Research, 2021
Background and Purpose: West syndrome is an epileptic encephalopathy of infancy. According to guidelines, adrenocorticotrophic hormone (ACTH) is probably effective for the short-term management of infantile spasm, but there is little uniformity in treatment due to variable response. This study has been done to evaluate the efficacy of pulse methylprednisolone as compared to ACTH in children with West syndrome.Methods: Children between 3 months to 24 months with the diagnosis of West syndrome were included and ACTH and pulse methyl prednisolone followed by oral prednisolone were given after randomization. Total duration of treatment was 6 weeks in both groups.Results: Total 87 children were enrolled; 12 patients lost in follow up. Finally, 43 received ACTH and 32 received pulse methylprednisolone. In pulse methylprednisolone group, 28.13% showed 50-80% response, 28.13% showed 80-99% response and 21.87% patients showed 100% response. In ACTH group, 41.86% showed 50-80% response, 25.58...
Cardiac Vagal Activation by Adrenocorticotropic Hormone Treatment in Infants with West Syndrome
The Tohoku Journal of Experimental Medicine, 2007
West syndrome (WS) is a generalized epileptic syndrome of infancy and early childhood with various etiologies, and consists of a triad of infantile spasm, arrest or regress of psychomotor development and specific electroencephalogram (EEG) pattern of hypsarrhythmia. WS had been believed to be refractory, but recent evidence supports effectiveness of adrenocorticotropic hormone (ACTH) treatment. The ACTH treatment, however, has a problem that it is often accompanied by adverse autonomic symptoms. We therefore examined heart rate variability (HRV) for assessing cardiac autonomic functions in WS and prospectively observed the changes during ACTH treatment. We studied 15 patients with WS and 9 age-matched controls during sleep (EEG stage 2). Compared with controls, the patients with WS were greater in the low-frequency component (LF) of HRV, an index reflecting sympatho-vagal interaction (p = 0.02), but were comparable for high-frequency component (HF) and LF-to-HF ratio (LF/HF), indices reflecting cardiac vagal activity and sympathetic predominance, respectively. During ACTH treatment, heart rate decreased (p < 0.01), LF and HF increased (p < 0.01), and LF/HF did not differ significantly. These results indicate that West syndrome might be accompanied by autonomic changes and that ACTH treatment enhances parasympathetic function and causes bradycardia. West syndrome; ACTH; heart rate variability; bradycardia; autonomic function